PEDIATRIC SURGERY UPDATE ©
VOLUME 21, 2003
Volume 21 No 01 JULY 2003
Bilateral Wilms Tumor
Synchronous bilateral Wilms tumor, also termed Stage V Wilms disease,
occurs in approximately 5% of all cases of Wilms tumor in children. Definitive
progress has been made during the past twenty years in diagnosis and management
of bilateral Wilms tumor with marked improvement in prognosis. Diagnosis
of bilaterality can be confirmed either most commonly during initial imaging
or rarely while performing exploratory laparotomy. Some of the better prognostic
factors associated with bilateral Wilms tumor are a patient age less than
three years at diagnosis, lower stage of the most advanced lesions, favorable
histology and negative nodal involvement. Most children (96%) with stage
V tumors have favorable histology. Currently, management of bilateral Wilms
tumor entails initial biopsy of both tumor masses, staging of lymph node
metastasis followed by preoperative chemotherapy. Cytoreductive chemotherapy
before surgical resection reduces tumor burden and permits more renal preservation
procedures. Jointly, renal salvage procedures (partial nephrectomy and
enucleation) have been recommended to conserve renal parenchyma. This has
facilitated the use of parenchymal-sparing operations, with the potential
advantage of decreasing the incidence of end-stage renal disease. Patients
with inoperable tumors and extensive intravascular tumor extension can
also benefit from this approach. Long-term follow-up reveals a group
of children who develops insidious renal compromise when more than 70%
parenchyma is compromise. Bilateral Wilms tumor with unfavorable histology
is associated with poor prognosis.
References:
1- Blute ML, Kelalis PP, Offord KP, Breslow N, Beckwith
JB, D'Angio GJ: Bilateral Wilms tumor. J Urol 138(4 Pt 2):968-73, 1987
2- Shaul DB, Srikanth MM, Ortega JA, Mahour GH: Treatment
of bilateral Wilms' tumor: comparison of initial biopsy and chemotherapy
to initial surgical resection in the preservation of renal mass and function.
J Pediatr Surg 27(8):1009-14, 1992
3- Ritchey ML, Coppes MJ: The management of synchronous
bilateral Wilms tumor. Hematol Oncol Clin North Am 9(6):1303-15, 1995
4- Horwitz JR, Ritchey ML, Moksness J, Breslow NE, Smith
GR, Thomas PR, Haase G, Shamberger RC, Beckwith JB: Renal salvage procedures
in patients with synchronous bilateral Wilms' tumors: a report from the
National Wilms' Tumor Study Group. J Pediatr Surg 31(8):1020-5, 1996
5- Ritchey ML: The role of preoperative chemotherapy
for Wilms' tumor: the NWTSG perspective.
National Wilms' Tumor Study Group. Semin Urol Oncol 17(1):21-7,
1999
6- Tomlinson GS, Cole CH, Smith NM: Bilateral Wilms'
tumor: a clinicopathologic review. Pathology 31(1):12-6, 1999
7- Fuchs J, Wunsch L, Flemming P, Weinel P, Mildenberger
H: Nephron-sparing surgery in synchronous bilateral Wilms' tumors. J Pediatr
Surg 34(10):1505-9, 1999
8- LinniPaya K, Horcher E, Lawrenz K, Rebhandl W, Zoubek
A: Bilateral Wilms' tumor--surgical aspects. Eur J Pediatr Surg 11(2):99-104,
2001
Crohn's Disease
Crohn's (terminal ileitis) is a chronic, transmural inflammatory bowel
disease most frequently involving the terminal ileum and proximal colon
that adversely affect growth and sexual maturation in children. Incidence
is growing and etiology is undetermined. Diarrhea, abdominal pain, failure
to thrive and weight loss are the most frequent clinical feature. Diagnosis
is established by colonoscopy or imaging studies (CT-Scan). Initial management
is medical and consists of azulfidine or 5-amino salicylic acid preparations,
local and systemic steroids, metronidazole, immunosuppressives, and enteral
and/or parenteral nutrition. Indication for surgery is limited to complications
of the disease process and includes failure of medical therapy, perforation,
abscess, severe malabsorption and growth retardation, persistent bowel
obstruction, fistulas (entero-enteric and entero-urinary) and strictures.
Surgery can be accomplished using limited resection and anastomosis or
stricturoplasty. Best long-term results after surgery occurs in children
with disease confine to the small bowel and ileocecal region. Diffuse ileocolonic
involvement (Panenteritis), preoperative use of 6-MP, and colonic involvement
is associated with early relapse. Early relapse after surgery is also seen
after failure of medical therapy independent of disease location as the
sole indication for surgery and in children undergoing resection within
one year of the onset of symptoms.
References:
1- Postuma R, Moroz SP: Pediatric Crohn's disease. J
Pediatr Surg 20(5):478-82, 1985
2- Davies G, Evans CM, Shand WS, Walker-Smith JA: Surgery
for Crohn's disease in childhood: influence of site of disease and operative
procedure on outcome. Br J Surg 77(8):891-4, 1990
3- Griffiths AM, Wesson DE, Shandling B, Corey M, Sherman
PM: Factors influencing postoperative recurrence of Crohn's disease in
childhood. Gut 32(5):491-5, 1991
4-Telander RL: Surgical management of Crohn's disease
in children. Curr Opin Pediatr 7(3):328-34, 1995
5- Patel HI, Leichtner AM, Colodny AH, Shamberger RC:
Surgery for Crohn's disease in infants and children. J Pediatr Surg 32(7):1063-7,
1997
6- Beattie RM: Therapy of Crohn's disease in childhood.
Paediatr Drugs 2(3):193-203, 2000
7- Baldassano RN, Han PD, Jeshion WC, Berlin JA, Piccoli
DA, Lautenbach E, Mick R, Lichtenstein GR: Pediatric Crohn's disease: risk
factors for postoperative recurrence. Am J Gastroenterol 96(7):2169-76,
2001
8- Dokucu AI, Sarnacki S, Michel JL, Jan D, Goulet O,
Ricour C, Nihoul-Fekete C: Indications and results of surgery in patients
with Crohn's disease with onset under 10 years of age: a series of 18 patients.
Eur J Pediatr Surg 12(3):180-5, 2002
Amniotic Band Syndrome
Amniotic or constricting band syndrome (ABS) refers to progressive intrauterine
amputation of fingers or limbs associated with a wide spectrum of congenital
anomalies involving the trunk and craniofacial region. Incidence is one
in 15,000 livebirths. Most amputations occur in the upper limb. In the
hand, digital amputations are most common in the index, middle, and ring
fingers, whereas in the foot, amputations of the hallux are most often
noted. The congenital (intrauterine) band, a product of rupture amnion,
produces compression and chronic ischemia of the affected limb. Multiple
anomalies are associated with most ABS cases such as orbital defects, lid
anomalies, lacrimal outflow obstruction, ocular malformations, waist constriction,
clubfoot, fascial cleft, cleft palate and lips. Follow-up ultrasound exams
have afforded the opportunity of observing the in utero process of limb
strangulation and subsequent spontaneous lysis of an amniotic band in a
few cases. Management depends on clinical findings at birth. Prognosis
depends on the severity of the abnormalities and the involvement of internal
organs.
References:
1- Light TR, Ogden JA: Congenital constriction band syndrome.
Pathophysiology and treatment. Yale J Biol Med 66(3):143-55, 1993
2- Froster UG, Baird PA: Amniotic band sequence and limb
defects: data from a population-based study. Am J Med Genet 46(5):497-500,
1993
3- Crombleholme TM, Dirkes K, Whitney TM, Alman B, Garmel
S, Connelly RJ: Amniotic band syndrome in fetal lambs. I: Fetoscopic release
and morphometric outcome. J Pediatr Surg 30(7):974-8, 1995
4- Bahadoran P, Lacour JP, Terrisse A, Ortonne JP: Congenital
constriction band of the trunk. Pediatr Dermatol 14(6):470-2, 1997
5- Bodamer OA, Popek EJ, Bacino C: Atypical presentation
of amniotic band sequence. Am J Med Genet 22;100(2):100-2, 2001
6- Muraskas JK, McDonnell JF, Chudik RJ, Salyer
KE, Glynn L: Amniotic band syndrome with significant orofacial clefts and
disruptions and distortions of craniofacial structures. J Pediatr Surg
38(4):635-638, 2003
Volume 21 No 02 AUGUST 2003
Ulcerative Colitis
Ulcerative colitis (UC) is a chronic debilitating inflammatory disease
of the bowel affecting primarily the mucosa and to a lesser extent to the
adjacent submucosa. UC affects mainly the rectum and colon causing delayed
growth and development. In some children the terminal ileum is affected.
Cause of UC remains unknown. Peak incidence is between 1.5 and 17 years
(mean 11 years). UC originates as an acute inflammation of the crypts (cryptitis)
developing tissue reaction of chronicity. More than 90% children with UC
have moderate to severe disease. Clinically, the child with UC develops
bloody diarrhea, abdominal cramps, anemia, fever, tachycardia, hypoalbuminemia
and weight loss. Colonoscopy is diagnostic. Medical management includes
restriction of milk protein from diet, parenteral nutrition, steroids,
sulfasalazine, metronidazole, 6-mercaptopurine, cyclosporine and tracolimus.
UC can be cured by surgical resection of the colon. Indications for surgery
in UC include inability to attain growth and development under medical
therapy, fulminant disease refractory to medical therapy, extensive rectal
bleeding, perforation and toxic megacolon. Surgical management consists
of total proctocolectomy and ileal pouch anal anastomosis. The J-pouch
is the simplest to construct. Retention of mucosa above the dentate line
after surgery produces recurrent inflammatory disease and high risk of
developing carcinoma. A low risk of bladder dysfunction and impotence due
to damage to pelvic nerves is associated with proctocolectomy. Postop complications
are associated with duration of the disease and length/dosage of medication
(steroids). Long-term function after surgery is good in more than 90% of
children with high patient satisfaction.
References:
1- Ament M, Vargas JH: Medical Therapy for Ulcerative
Colitis in Childhood. Semin Pediatr Surg 3(1): 28-32, 1994
2- Fonkalsrud EW: Surgical management of ulcerative colitis
in childhood. Semin Pediatr Surg 3(1):33-8, 1994
3- Nicholls S, Vieira MC, Majrowski WH, Shand WS, Savage
MO, Walker-Smith JA: Linear growth after colectomy for ulcerative colitis
in childhood. J Pediatr Gastroenterol Nutr 21(1):82-6, 1995
4- Rintala RJ, Lindahl H: Restorative proctocolectomy
for ulcerative colitis in children--is the J-pouch
better than straight pull-through? J Pediatr Surg 31(4):530-3,
1996
5- Hyams JS, Davis P, Grancher K, Lerer T, Justinich
CJ, Markowitz J: Clinical outcome of ulcerative colitis in children. J
Pediatr 129(1):81-8, 1996
6- Durno C, Sherman P, Harris K, Smith C, Dupuis A, Shandling
B, Wesson D, Filler R, Superina R, Griffiths A: Outcome after ileoanal
anastomosis in pediatric patients with ulcerative colitis. J Pediatr Gastroenterol
Nutr 27(5):501-7,1998
7- Dolgin SE, Shlasko E, Gorfine S, Benkov K, Leleiko
N: Restorative proctocolectomy in children with ulcerative colitis utilizing
rectal mucosectomy with or without diverting ileostomy. J Pediatr Surg
34(5):837-9, 1999
8- Fonkalsrud EW, Thakur A, Beanes S: Ileoanal pouch
procedures in children. Pediatr Surg 36(11):1689-92, 2001
9- Rintala RJ, Lindahl HG: Proctocolectomy and J-pouch
ileo-anal anastomosis in children. J Pediatr Surg 37(1):66-70, 2002
10- Mahadevan U, Loftus EV Jr, Tremaine WJ, Pemberton
JH, Harmsen WS, Schleck CD, Zinsmeister AR, Sandborn WJ: Azathioprine or
6-mercaptopurine before colectomy for ulcerative colitis is not associated
with increased postoperative complications. Inflamm Bowel Dis 8(5):311-6,
2002
Carotid Body Tumor
Chemodectomas (tumors of chemoreceptors cells origin) are called carotid
body tumors when they occur in the carotid artery, and glomus tumors if
they appear in the jugular vein. Sex distribution for the two major types
is equal for males and females. A few cases have been reported in the pediatric
age. They are associated with neck swelling and hypertension. Carotid body
tumors (CBT) are extra-adrenal paragangliomas diagnosed early in life which
can have familial inheritance. Familial cases are of autosomic dominant,
bilateral in location and multicentric. Diagnostic work-up includes angiography,
CT and MRI. Surgical excision is the treatment of choice for CBT and glomus
tumors. Almost three-fourth of CBT and cervical paragangliomas are adherent
to or surround adjacent arteries and cranial nerves. Their resection can
result in neurovascular injury, stroke and excessive blood loss.
References:
1- Parry DM, Li FP, Strong LC, Carney JA, Schottenfeld
D, Reimer RR, Grufferman S: Carotid body tumors in humans: genetics and
epidemiology. J Natl Cancer Inst 68(4):573-8, 1982
2- Dickinson PH, Griffin SM, Guy AJ, McNeill IF: Carotid
body tumour: 30 years experience. Br J Surg 73(1):14-6, 1986
3- Bishop GB Jr, Urist MM, el Gammal T, Peters GE, Maddox
WA: Paragangliomas of the neck. Arch Surg 127(12):1441-5, 1992
4- Varudkar AS, Kokandkar HR, Gumaste GG, Bhople KS,
Kumbhakarna NR: Carotid body paraganglioma with coexistent pheochromocytoma
in childhood. Indian J Cancer 30(3):109-12, 1993
5- Plukker JT, Brongers EP, Vermey A, Krikke A, van den
Dungen JJ: Outcome of surgical treatment for carotid body paraganglioma.
Br J Surg 88(10):1382-6, 2001
Perianal Dermatitis
Diaper dermatitis is a group of skin disorders resulting from attack
of the skin by physical, chemical, enzymatic, and microbial factors in
the diaper environment. Perineal or diaper dermatitis after colo-anal surgical
procedures can be a troublesome condition in children. The two most common
procedures associated with perianal dermatitis are pull-through for Hirschsprung's
disease and to a lesser extent following repair of imperforate anus. The
main factors associated with this medical problem are the muscular pseudo
incontinence associated with the procedure, frequent bowel movements, postoperative
diarrhea and the alkali milieu in contact with the perianal skin. The rash
can include mild redness, skin excoriation, pseudo verrucous papules and
nodules depending on length of time of skin irritative contact. Histology
shows benign epidermal hyperplasia, reactive acanthosis or psoriasiform
spongiotic dermatitis. Managing this condition can be very challenging.
This includes water barrier agents, local therapy (A&D, nystatin, zinc
oxide), binding agents (cholestyramine) and anti-diarrhea medication. The
lesions regress when the irritating factor is removed. A novel approach
to reduce the perianal diaper rash associated after closure of colostomy
in infants is to paint the perianal skin with the liquid effluent of the
colostomy at least two weeks prior to the intended procedure. This permits
the perianal skin and proprioception reflex mechanism to adjust to
the external skin milieu after closure of a colostomy.
References:
1- Rodriguez Cano L, Garcia-Patos Briones V, Pedragosa
Jove R, Castells Rodellas A: Perianal pseudo verrucous papules and nodules
after surgery for Hirschsprung disease. J Pediatr 125(6 Pt 1):914-6, 1994
2- Bourrat E, Vaquin C, Prigent F, Rybojad M: Perianal
papulonodular dermatitis in Hirschsprung disease. Ann Dermatol Venereol
123(9):549-51, 1996
3- Goldberg NS, Esterly NB, Rothman KF, Fallon JD, Cropley
TG, Szaniawski W, Glassman M: Perianal pseudo verrucous papules and nodules
in children. Arch Dermatol 128(2):240-2, 1992
4- Hanlon M, Cofone E: Patient with frequent liquid stools
resulting in a chemical dermatitis and a perianal ulcer. J Wound Ostomy
Continence Nurs 23(3):174-7, 1996
5- Berg RW: Etiology and pathophysiology of diaper dermatitis.
Adv Dermatol 3:75-98, 1988
VOLUME 21 No 03 SEPTEMBER 2003
Ectopic Thymus
Finding ectopic thymus in a cervical mass is a rare diagnosis found
sporadically in infants and children. Cervical thymic lesions can either
be symptomless or cause severe dyspnea and dysphagia, especially in the
young infant. Aberrant migration of thymic tissue occurs with ectopic thymus
in the superior & posterior mediastinum, bases of the skull, tracheal
bifurcation, and cervical region. Aberrant ectopic thymic tissue can present
as either a solid or cystic mass in the neck of the child. Cervical location
(85%) and cystic nature (70%) is found more commonly. Occasionally, parathyroid
glands have been associated with the thymic remnants. Most cases remain
asymptomatic. The thymus is a paired organ which develops from the 3rd
and 4th pharyngeal pouch and descends into the superior mediastinum between
the 6th and 12th weeks of fetal development. Failure of the unilateral
gland to descend explains the finding of the ectopic cervical thymic tissue.
Another pathogenetic mechanism includes sequestration of accessory cervical
foci of thymic tissue along the normal cervical pathway of descend. A normal
chest-x-ray without evidence of absent thymic shadows suggests this mechanism
of sequestration. The most common cervical location is along the
anterior border of the sternocleidomastoid muscle lateral to the thyroid
gland and near the carotid sheath. Malignant transformation of ectopic
thymus tissue has been documented. Diagnosis is rarely done preoperatively.
Management consists of complete surgical excision. Symptoms due to pressure
on neighboring structures are promptly eliminated after excision. Prognosis
is excellent.
References:
1- Tovi F, Mares AJ: The aberrant cervical thymus. Embryology,
Pathology, and clinical implications. Am J Surg 136(5):631-7, 1978
2- Spigland N, Bensoussan AL, Blanchard H, Russo P: Aberrant
cervical thymus in children: three case reports and review of the literature.
J Pediatr Surg 25(11):1196-9, 1990
3- Gimm O, Krause U, Wessel H, Finke R, Dralle H: Ectopic
intrathyroidal thymus diagnosed as a solid thyroid lesion: case report
and review of the literature. J Pediatr Surg 32(8):1241-3, 1997
4- Krysta MM, Gorecki WJ, Miezynski WH: Thymic tissue
manifesting as a posterior mediastinal mass in two children. J Pediatr
Surg 33(4):632-4, 1998
5- Terzakis G, Louverdis D, Vlachou S, Anastasopoulos
G, Dokianakis G, Tsikou-Papafragou A: Ectopic thymic cyst in the neck.
J Laryngol Otol 114(4):318-20, 2000
6- Bernig T, Weigel S, Mukodzi S, Beck JF, Wiersbitzky
H, von Suchodoletz H, Warzok R: Ectopic cervical thymus in a 12-year-old
boy: a case report. Pediatr Hematol Oncol 17(8):713-7, 2000
7- Saggese D, Ceroni Compadretti G, Cartaroni C: Cervical
ectopic thymus: a case report and review of the literature. Int J Pediatr
Otorhinolaryngol 66(1):77-80, 2002
Mesenteric Adenitis
Mesenteric lymphadenitis is the condition most commonly mimicking
acute appendicitis resulting in a high rate of negative appendectomies
in children. Mesenteric adenitis is frequently associated with an upper
respiratory infection. Clinical presentation includes fever, leukocytosis
and low abdominal pain. Mesenteric adenitis can be the result of a viral
or bacterial infection. Viruses implicated includes Epstein-Barr, Adenovirus
type 3, influenza B and Coxsackie B. Bacteria associated with mesenteric
adenitis includes hemolytic streptococci, Yersinia and Salmonella species.
The diagnosis of mesenteric adenitis is principally one of exclusion. CT-Scan
can help decide whether the child has mesenteric adenitis when the lymph
nodes aggregates can be clearly seen. Otherwise, since it can be very difficult
to distinguish appendicitis from mesenteric adenitis the diagnosis is establish
at surgery. Laparoscopy can also be useful to differentiate appendicitis
from mesenteric adenitis. After surgery the postoperative course of children
with mesenteric adenitis is usually uneventful and recovery is rapid.
References:
1- Alvear DT, Kain TM 3rd: Suppurative mesenteric lymphadenitis,
a forgotten clinical entity: report of two cases. J Pediatr Surg 10(6):969-70,
1975
2- Achong DM, Oates E, Harris B: Mesenteric lymphadenitis
depicted by indium 111-labeled white blood cell imaging. J Pediatr Surg
28(12):1550-2, 1993
3- Macari M, Hines J, Balthazar E, Megibow A: Mesenteric
adenitis: CT diagnosis of primary versus secondary causes, incidence, and
clinical significance in pediatric and adult patients. AJR Am J Roentgenol
178(4):853-8, 2002
4- Arda IS, Ergin F, Varan B, Demirhan B, Aslan H, Ozyaylali
I: Acute abdomen caused by Salmonella typhimurium infection in children.
J Pediatr Surg 36(12):1849-52, 2001
5- Gilmore OJ, Browett JP, Griffin PH, Ross IK, Brodribb
AJ, Cooke TJ, Higgs MJ, Williamson RC: Appendicitis and mimicking conditions.
A prospective study. Lancet 2(7932):421-4, 1975
Esophageal Elongation
One unresolved surgical problem in pediatrics deals with babies born
with esophageal atresia and a long segment between the esophageal stumps
enabling primary anastomosis. Long gap esophageal atresia includes stumps
at least three vertebral bodies apart (approximately 3 cm in length)
or longer. Many techniques have been developed to deal with this problem
such as proximal esophageal stump dilatation, waiting for the stump to
grow spontaneously with time, use of myotomies, multistage extrathoracic
esophageal elongation, elongation of the lesser curvature, replacement
of esophagus with stomach, jejunum or colon to mention a few. Consensus
between pediatric surgeons worldwide is that there is no better esophageal
substitute in a child than the native esophagus. Other authors have found
that infants with an exclusive intraabdominal pouch will not reach sufficient
elongation and should be considered early as a candidate for esophageal
replacement. A recent innovative technique described by Foker using external
traction sutures in the esophageal ends have demonstrated to produce sufficient
lengthening within 6-10 days for a true primary anastomosis to be accomplished.
References:
1- Kimura K, Soper RT: Multistaged extrathoracic esophageal
elongation for long gap esophageal atresia. J Pediatr Surg 29(4):566-8,
1994
2- Fernandez MS, Gutierrez C, Ibanez V, Lluna J, Barrios
JE, Vila JJ, Garcia-Sala C: Long-gap esophageal atresia: reconstruction
preserving all portions of the esophagus by Scharli's technique. Pediatr
Surg Int 14(1-2):17-20, 1998
3- Maksoud-Filho JG, Goncalves ME, Tannuri U, Maksoud
JG: An exclusively intraabdominal distal esophageal segment prevents primary
delayed anastomosis in children with pure esophageal atresia. J Pediatr
Surg 37(11):1521-5, 2002
4- Boyle EM Jr, Irwin ED, Foker JE: Primary repair
of ultra-long-gap esophageal atresia: results without a lengthening procedure.
Ann Thorac Surg 57(3):576-9, 1994
5- Foker JE, Linden BC, Boyle EM Jr, Marquardt C: Development
of a true primary repair for the full spectrum of esophageal atresia. Ann
Surg 226(4):533-41, 1997
6- Gaglione G, Tramontano A, Capobianco A, Mazzei S:
Foker's technique in oesophageal atresia with double fistula: a case report.
Eur J Pediatr Surg 13(1):50-3, 2003
7- Al-Qahtani AR, Yazbeck S, Rosen NG, Youssef S, Mayer
SK: Lengthening technique for long gap esophageal atresia and early anastomosis.
J Pediatr Surg 38(5):737-9, 2003
VOLUME 21 No 04 OCTOBER 2003
Thyroid Cysts
Pediatric thyroid nodules are a source of concern for physicians as
they can harbor a malignancy. Initial work-up should include neck ultrasonography
to define anatomic location and determine whether we are dealing with a
cystic, solid or mixed lesion. Cystic and mixed solid-cystic thyroid masses
in children are most commonly benign lesions thought to arise from necrosis
and degeneration of thyroid nodules. In a few cases (8%) a malignancy can
present as a cystic lesion. Next step in management of a cystic thyroid
lesion is fine-needle aspiration cytology to establish a diagnosis. Unfortunately,
needle aspiration has yield false-negative results in patients with cystic
papillary carcinomas. The cysts in patients with cancer appear to originate
from necrosis of tumors measuring between two and 4 cm in diameter. Ethanol
or tetracycline sclerotherapy has been found safe and effective in the
management of thyroid cysts. Pain and drunken feeling are side effects
of ethanol sclerotherapy. Fearfully, you could be also sclerosing a hidden
papillary carcinoma. The most definitive management of thyroid cysts is
surgical excision. Thyroid lobectomy harboring the cyst should be performed
to children demonstrating probable or proven cytologic malignant changes
and those with recurrence of the cyst after serial aspiration and suppressive
therapy. Other factors such as size (greater than 3 cm in diameter),
history of neck irradiation or family thyroid cancer, and cervical lymphadenopathy
should be given weight in favor of surgical resection.
References:
1- Lugo-Vicente H, Ortiz VN, Irizarry H, Camps JI, Pagan
V: Pediatric thyroid nodules: management in the era of fine needle aspiration.
J Pediatr Surg 33(8):1302-5, 1998
2- Desjardins JG, Khan AH, Montupet P, Collin PP, Leboeuf
G, Polychronakos C, Simard P, Boisvert J, Dube LJ: Management of thyroid
nodules in children: a 20-year experience. J Pediatr Surg 22(8):736-9,
1987
3- Muller N, Cooperberg PL, Suen KC, Thorson SC: Needle
aspiration biopsy in cystic papillary carcinoma of the thyroid. AJR Am
J Roentgenol 144(2):251-3, 1985
4- Hammer M, Wortsman J, Folse R: Cancer in cystic lesions
of the thyroid. Arch Surg 117(8):1020-3, 1982
5- Rosen IB, Provias JP, Walfish PG: Pathologic nature
of cystic thyroid nodules selected for surgery by needle aspiration biopsy.
Surgery 100(4):606-13, 1986
6- Sarda AK, Bal S, Dutta Gupta S, Kapur MM: Diagnosis
and treatment of cystic disease of the thyroid by aspiration. Surgery 103(5):593-6,
1988
7- Yoskovitch A, Laberge JM, Rodd C, Sinsky A, Gaskin
D: Cystic thyroid lesions in children. J Pediatr Surg 33(6):866-70, 1998
Cervical Clefts
Congenital clefts can rarely occur in the face or the neck of a child.
Fascial cleft, also known as congenital macrostomia, is a transverse deformity
developing from the first and second branchial arches. Cervical clefts
are almost always midline in location. This rare developmental anomaly
represents failure of the branchial arches to fuse in the midline and presents
at birth with a ventral midline defect of the skin of the neck extending
for a variable distance from the chin to the suprasternal notch. Most cases
reported are white females. Inially the cleft is covered by an exudative
thin desquamating epithelium which toughens and dries during the following
weeks creating scarring and contracture. The covering epithelium lacks
sweat glands, sebaceous glands or hair follicles. The cranial end of the
cleft has a nipple-like protuberance while the caudal end presents as an
opening to a sinus tract where mucoid secretions can be seen. The mucoid
discharge is the product of ectopic salivary glands. Beneath the cleft
there is a firm submucosal fibrous cord. Differential diagnosis includes
branchial cleft anomaly, thyroglossal duct cysts (or fistula) and ectopic
bronchogenic cysts. Occasionally, associated heart lesions have been described.
Unlike thyroglossal duct cysts, midline cervical cleft has no anatomical
association with the hyoid bone. In a few cases a bony prominence of the
mandible is palpable and seen as a spur in x-ray films. The spur is due
to traction of the fibrous cord on the bone. Management consists of complete
excision of all pathologic tissue along with the underlying cord. The wound
can be closed primarily using a z-plasty technique. Early surgery avoids
neck contracture and deformity of the mandible.
References:
1- Maschka DA, Clemons JE, Janis JF: Congenital midline
cervical cleft. Case report and review. Ann Otol Rhinol Laryngol 104(10
Pt 1):808-11, 1995
2- Hirokawa S, Uotani H, Okami H, Tsukada K, Futatani
T, Hashimoto I: A case of congenital midline cervical cleft with congenital
heart disease. J Pediatr Surg 38(7):1099-101, 2003
3- Ayache D, Ducroz V, Roger G, Garabedian EN: Midline
cervical cleft. Int J Pediatr Otorhinolaryngol 20;40(2-3):189-93, 1997
4- van der Staak FH, Pruszczynski M, Severijnen RS, van
de Kaa CA, Festen C: The midline cervical cleft. J Pediatr Surg 26(12):1391-3,
1991
5- Eastlack JP, Howard RM, Frieden IJ: Congenital midline
cervical cleft: case report and review of the English language literature.
Pediatr Dermatol 17(2):118-22, 2000
6- Bergevin MA, Sheft S, Myer C 3rd, McAdams AJ: Congenital
midline cervical cleft. Pediatr Pathol 9(6):731-9, 1989
7- Gargan TJ, McKinnon M, Mulliken JB: Midline cervical
cleft. Plast Reconstr Surg 76(2):225-9, 1985
Eosinophilic Granuloma
Langerhans cell histiocytosis, also known as eosinophilic granuloma,
is a localized benign tumor seen in bones, skull, ribs, spine, pelvis and
scalp area. It is estimated that 7% of all scalp lesions in children are
eosinophilic granulomas. Eosinophilic granuloma arises from an abnormal
proliferation of histiocytes. Children develop the lesions during the first
decade of life manifesting pain, tenderness and swelling of the affected
areas. Males are affected twice as much as females. The clinical course
for most patients is benign depending on the location of the lesion. Simple
X-ray of the lesion will show a lytic, well-defined "punched-out" lesion
in bone with marginal reactive sclerosis. CT-Scan will describe the extent
of the disease process. Biopsy is imperative to establish a histologic
diagnosis. Management consists of observation alone, curettage, low-dose
radiation therapy or intralesional injection of steroids.
References:
1- Appling D, Jenkins HA, Patton GA: Eosinophilic granuloma
in the temporal bone and skull. Otolaryngol Head Neck Surg 91(4):358-65,
1983
2- Ruge JR, Tomita T, Naidich TP, Hahn YS, McLone DG:
Scalp and calvarial masses of infants and children. Neurosurgery 22(6 Pt
1):1037-42, 1988
3- Greis PE, Hankin FM: Eosinophilic granuloma. The management
of solitary lesions of bone. Clin Orthop (257):204-11, 1990
4- Martinez-Lage JF, Poza M, Cartagena J, Vicente JP,
Biec F, de las Heras M: Solitary eosinophilic granuloma of the pediatric
skull and spine. The role of surgery. Childs Nerv Syst 7(8):448-51, 1991
5- Plasschaert F, Craig C, Bell R, Cole WG, Wunder JS,
Alman BA: Eosinophilic granuloma. A different behaviour in children than
in adults. J Bone Joint Surg Br 84(6):870-2, 2002
Volume 21 No 05 NOVEMBER 2003
Lymphangiomas - Fibrin Seal
Lymphangiomas are congenital cystic tumors developing in the neck, axilla,
chest and trunk. Characteristically they are composed of multiple sacs
or loculation of lymphatic fluid. Cysts can vary in size from microcysts
to large cystlike dilatations. The tumor can compress vital structure.
The close relation of this benign tumor with vital structures reduces the
chances of complete surgical excision in many cases. This has brought forth
the need for alternative therapy such as sclerotherapy. Two such agents
in use today for sclerosing lymphangiomas are OK-432 and fibrin glue sealant.
OK-432 has not yet been approved by FDA. In 1988 fibrin glue successfully
sealed a postoperative persistent lymphatic drainage in a chid after persistent
drainage. Fibrin glue or sealant, an FDA approved product, consists of
a mixture of fibrinogen, thrombin, a fibrinolysis inhibitor and calcium
in separate vials. When mixed they form a viscous solution that sets into
an elastic coagulum. Thrombin transforms fibrinogen into fibrin. The inhibitor
prevents premature degradation of fibrin. Fibrin glue is adhesive, it is
locally hemostatic and it regenerates tissue that favor the synthesis of
collagen. The adhesive can effectively seal tissues surfaces and eliminate
potential dead spaces. The technique for fibrin seal of lymphangiomas consists
in percutaneous puncture of the cyst and aspiration of the whole content
followed by introduction of fibrin sealant into the cavity. The amount
injected consist of 10 to 15% of the suctioned volume. Treatment can be
repeated. Results are very encouraging with few minor side effects such
as erythema and cellulitis.
References:
1- Giberson WG, McCarthy PM, Kaufman BH: Fibrin glue
for the treatment of persistent lymphatic drainage. J Pediatr Surg 23(12):1188-9,
1988
2- Castanon Garcia-Alix M, Margarit Mallol J, Martin
Hortiguela ME, Salarich de Arbell J: [Fibrin adhesive: a new therapeutic
alternative in the treatment of cystic lymphangioma]. An Esp Pediatr 38(4):304-6,
1993
3- Gutierrez San Roman C, Barrios J, Lluna J, Menor F,
Poquet J, Ruiz S: Treatment of cervical lymphangioma using fibrin adhesive.
Eur J Pediatr Surg 3(6):356-8, 1993
4- Castanon Garcia-Alix M, Margarit Mallol J, Garcia
Baglietto A, Martin Hortiguera ME, Morales Fochs L: [Cystic lymphangioma:
treatment with adhesive fibrin tissue. Follow-up study]. Cir Pediatr Jan;9(1):36-9,
1996
5- Castanon M, Margarit J, Carrasco R, Vancells M, Albert
A, Morales L: Long-term follow-up of nineteen cystic lymphangiomas treated
with fibrin sealant. J Pediatr Surg 34(8):1276-9, 1999
6- Honig JF, Merten HA: Surgical removal of intra- and
extraoral cavernous lymphangiomas using intraoperative-assisted intralesional
fibrin glue injections. J Craniofac Surg 11(1):42-5, 2000
Abdominal Incisions
Pediatric surgeons utilize several types of abdominal incision to approach
different surgical problems in newborns, infants and children. In most
children and during the first five years of life transverse incisions are
preferred. It has been demonstrated that the younger the child, the relatively
larger the abdominal cavity and wall. Because of the anatomical differences
between the abdomen of adults and small children, the cavity of the pediatric
patient resembles a horizontally oriented ellipsoid. Being barrel-shaped
a transverse incision provides better exposure to all four quadrants in
young children. The younger the child, the larger proportionately is the
costo-iliac space, allowing for easier lateral extension of the incision.
In babies a supraumbilical transverse incision is ideal to explore all
four quadrants and solve almost every surgical congenital abdominal condition.
Another advantage of transverse incision over longitudinal incision is
the low incidence of fascial dehiscence, hernia formation, and evisceration
of transverse incisions. Whether to use mass fascial closure or layer closure
no significant statistic difference in rate of complications has been identified
in abdominal incisions in children. Finally, transverse closure carries
better cosmetic results than vertical incision. In older children a midline
incision is still the incision of choice in conditions that require rapid
intra-abdominal entry such as trauma.
References:
1- Gauderer MW: A rationale for routine use of transverse
abdominal incisions in infants and children. J Pediatr Surg 16(4 Suppl
1):583-6, 1981
2- Ellis H, Coleridge-Smith PD, Joyce AD: Abdominal incisions--vertical
or transverse? Postgrad Med J 60(704):407-10, 1984
3- Chana RS, Saxena VC, Agarwal A: A prospective study
of closure techniques of abdominal incisions in infants and children. J
Indian Med Assoc 91(3):59-61, 1993
4- Waldhausen JH, Davies L: Pediatric postoperative abdominal
wound dehiscence: transverse versus vertical incisions. J Am Coll Surg
190(6):688-91, 2000
5- Grantcharov TP, Rosenberg J: Vertical compared with
transverse incisions in abdominal surgery. Eur J Surg 167(4):260-7, 2001
Myoblastoma
Granular cell tumor (GCT), also known as myoblastoma, is a lesion of
unknown etiology and histogenesis rarely found in children. GTC most commonly
appear in the skin or soft tissue of the trunk and extremity during the
life of a child. Other sites described includes the oral cavity (tongue,
gingiva, trachea, larynx), esophagus, breast, perineum and parotid gland.
A preference for the Negro race has been identified in several series.
Most GCT are benign single lesions. A few patients develop multiple benign
lesions. GCT tends to recur locally. The cytopathologic features of GCT
are distinctive enough to allow a correct diagnosis using FNA cytology.
The malignant variant of granular cell tumor is a high-grade sarcoma with
a high rate of metastases and a short survival. Management of benign GCT
consists of local excision leaving margins free of tumor. Very rarely GCT
has been reported to metastasize to the lung.
References:
1- Apisarnthanarax P: Granular cell tumor. An analysis
of 16 cases and review of the literature. J Am Acad Dermatol 5(2):171-82,
1981
2- Morrison JG, Gray GF Jr, Dao AH, Adkins RB Jr: Granular
cell tumors. Am Surg 53(3):156-60, 1987
3- Fanburg-Smith JC, Meis-Kindblom JM, Fante R, Kindblom
LG: Malignant granular cell tumor of soft tissue: diagnostic criteria and
clinicopathologic correlation. Am J Surg Pathol 22(7):779-94, 1998
4- Billeret Lebranchu V: [Granular cell tumor. Epidemiology
of 263 cases]. Arch Anat Cytol Pathol 47(1):26-30, 1999
5- Mallik MK, Das DK, Francis IM, al-Abdulghani R, Pathan
SK, Sheikh ZA, Luthra UK: Fine needle aspiration cytology diagnosis of
a cutaneous granular cell tumor in a 7-year-old child. A case report. Acta
Cytol 45(2):263-6, 2001
Volume 21 No 06 DECEMBER 2003
Recurrent Inguinal Hernias
Inguinal hernias continue to be the most common congenital pathology
in children needing surgical repair early in life. Approximately 1-3% of
children have an inguinal hernia. The incidence is higher in premature
babies (3-5%). Almost all inguinal hernias in children are the indirect
type (99%). The few direct hernias in children are the result of previous
surgery or inguinal floor disruption. Management of inguinal hernias in
infants and children is straightforward: outpatient surgery after diagnosis
for most cases. The procedure consists of high ligation of the hernial
sac. Incidence of developing a recurrent inguinal hernia is around 0.8%.
Most recurrences occur two years after the initial surgery. Several factors
play a role in increasing hernia recurrence. These are: 1) Missed sac or
inadequate ligation of the indirect sac. 2) Children operated for incarcerated
inguinal hernias since tissue is more friable and edematous at the time
of surgery. 3) Infection of the wound after hernia repair predisposing
to tissue breakdown and a higher recurrence rate. 4) Connective tissue
disorders (Hurler, Ehlers-Danlos, etc.). 5) Growth failure and poor nutrition.
6) Prematurity has been identified as a co-morbid factor in hernia recurrence.
7) Children hernia repair done by non-pediatric surgeons is also a risk
factor for recurrence. 8) Conditions causing increase intra-abdominal pressure
(VP shunts, posterior urethral valves, bladder exstrophy repair, weight
lifting and respiratory conditions) are also related to higher rate of
recurrence in children. Repair of the recurrent hernia is done through
the inguinal scar or using laparoscopy. The sac is mobilized and ligated.
Laparoscopic repair of recurrent inguinal hernia has the advantage of passing
through a virgin field reducing damage to vas or vessels and allowing inspection
of the area with direct purse string repair of the defect.
References:
1- Grosfeld JL, Minnick K, Shedd F, West KW, Rescorla
FJ, Vane DW: Inguinal hernia in children: factors affecting recurrence
in 62 cases. J Pediatr Surg 26(3):283-7, 1991
2- Steinau G, Treutner KH, Feeken G, Schumpelick V: Recurrent
inguinal hernias in infants and children. World J Surg 19(2):303-6, 1995
3- Esposito C, Montupet P: Laparoscopic treatment of
recurrent inguinal hernia in children. Pediatr Surg Int 14(3):182-4, 1998
4- Perlstein J, Du Bois JJ: The role of laparoscopy in
the management of suspected recurrent pediatric hernias. J Pediatr Surg
35(8):1205-8, 2000
5- Meier AH, Ricketts RR: Surgical complications of inguinal
and abdominal wall hernias. Semin Pediatr Surg 12(2):83-88, 2003
Incarcerated Inguinal Hernias
Incarceration and strangulation are the most dreaded complications of
inguinal hernias in children. Incarceration refers to viscera (bowel, ovaries,
bladder) that protrudes through the inguinal defect and cannot return back
to its anatomical position without manipulation or surgery. Bowel incarceration
in infants with inguinal hernia is a notable cause of intestinal obstruction
in this age group. Strangulation is the ischemic effect caused on the trapped
viscera by the incarcerated defect. Incarceration occurs in almost one-third
of inguinal hernias. It is more common in children less than one year of
age and males. With prolonged incarceration there can also occur testicular
infarction. In infant girls the normal anatomy is altered when an ovary
is trapped in a hernia sac, and these changes make torsion more likely.
This risk warrants treating the asymptomatic irreducible ovary as any other
incarcerated hernia with urgency. Incarceration increases the rate of complications,
is seen in a younger population of children and increases hospital stay.
Children with incarcerated hernia should have a trial of manual reduction
followed by prompt repair within the next five to seven days to avoid re-incarceration.
References:
1- Palmer BV: Incarcerated inguinal hernia in children.
Ann R Coll Surg Engl 60(2):121-4, 1978
2- Puri P, Guiney EJ, O'Donnell B: Inguinal hernia in
infants: the fate of the testis following incarceration. J Pediatr Surg
19(1):44-6, 1984
3- Boley SJ, Cahn D, Lauer T, Weinberg G, Kleinhaus S:
The irreducible ovary: a true emergency. J Pediatr Surg 26(9):1035-8, 1991
4- Stephens BJ, Rice WT, Koucky CJ, Gruenberg JC: Optimal
timing of elective indirect inguinal hernia repair in healthy children:
clinical considerations for improved outcome. World J Surg 16(5):952-6,
1992
5- Stylianos S, Jacir NN, Harris BH: Incarceration of
inguinal hernia in infants prior to elective repair. J Pediatr Surg 28(4):582-3,
1993
6- Gahukamble DB, Khamage AS: Early versus delayed repair
of reduced incarcerated inguinal hernias in the pediatric population. J
Pediatr Surg 31(9):1218-20, 1996
7- Niedzielski J, Kr l R, Gawlowska A: Could incarceration
of inguinal hernia in children be prevented? Med Sci Monit 9(1):CR16-8,
2003
Acute Chest Syndrome
Acute chest syndrome (ACS) is a pneumonia like illness, the most frequent
cause of hospitalization and morbidity in children with Sickle Cell Disease
(SCD). ACS can also occur after surgical procedures in children with SCD.
Clinically ACS includes fever, cough, chest pain, leukocytosis and a new
infiltrate in chest films. The most common etiologic factors for ACS include
infection, pulmonary fat embolism (infarcted bone marrow) and hypoventilation.
History of pulmonary disease is a predictive factor for the ACS. The risk
of ACS is inversely proportional to age with the highest incidence in small
children. Abdominal surgery is a high risk situation to develop postoperative
ACS with characteristic basilar atelectasis after either open or laparoscopic
surgery. Preoperative transfusion reduces pulmonary complications and is
beneficial. Management of ACS encompasses antibiotics, hydration, oxygenation,
transfusion, analgesia, bronchodilators, supportive respiratory therapy
and antiinflammatory agents.
References:
1- Ware RE, Filston HC: Surgical management of children
with hemoglobinopathies. Surg Clin North Am 72(6):1223-36, 1992
2- Castro O, Brambilla DJ, Thorington B, Reindorf CA,
Scott RB, Gillette P, Vera JC, Levy PS: The acute chest syndrome in sickle
cell disease: incidence and risk factors. The Cooperative Study of Sickle
Cell Disease. Blood 15;84(2):643-9, 1994
3- Dreyer ZE: Chest infections and syndromes in sickle
cell disease of childhood. Semin Respir Infect 11(3):163-72, 1996
4- Vichinsky EP, Neumayr LD, Earles AN, Williams R, Lennette
ET, Dean D, Nickerson B, Orringer E, McKie V, Bellevue R, Daeschner C,
Manci EA: Causes and outcomes of the acute chest syndrome in sickle cell
disease. National Acute Chest Syndrome Study Group. N Engl J Med 342(25):1855-65,
2000
5- Wales PW, Carver E, Crawford MW, Kim PC: Acute chest
syndrome after abdominal surgery in children with sickle cell disease:
Is a laparoscopic approach better? J Pediatr Surg 36(5):718-21, 2001
6- Siddiqui AK, Ahmed S: Pulmonary manifestations of
sickle cell disease. Postgrad Med J 79(933):384-90, 2003